Microvascular occlusion syndromes Flashcards
1
Q
HITS epi
A
- 10X higher in unfractionated heparin compared with LMWH
- F>M
- rare in pregnancy
2
Q
HITs pathogenesis
A
- Platelet 4 factor is released and complexes with heparin –> rapid IgG antibody response within 5-7 days develops
- Immune complexes develop –> activating platelets and monocytes
- These activated platelets aggregate and produce platelet microparticles which generate thrombin, and thrombocytopaenia develops
- Activated monocytes and endothelial cells also increase their expression of tissue factor –> thrombin production and vascular occlusion
3
Q
HITS clinical
A
- Timing
- 5-10 days after heparin starts
- if have had heparin in the last 90-100 days, then HITS can develop within hours after re-exposure
- rarely can have anaphylactoid reaction within 30 minutes of a heparin bolus
- Delayed-onset: lesions can occur up to 3 weeks after exposure to heparin
- Atypical scenarios:
- Chronic heparin –> can trigger within 5-10 days
- sometimes without heparin, major surgery or bacterial infections can trigger a spontaneous or autoimmune HIT syndrome
- 5-10 days after heparin starts
- Cutaneous
- Tender, sharply demarcated, non-inflammatory and purpuric or necrotic, retiform
- Can occur at sites of S/C injection, or distant to heparin infusion
- Platelets
- Don’t often get thrombocytopaenia on bloods, although there is a consumption of platelets
- Decline in platelet of >50% from highest count should prompt consideration
- Cx: vascular occlusion to any limb or organ
4
Q
HITS pathology
A
non-inflammatory occlusion of blood vessels and ischaemic necrosis
5
Q
HITS dx
A
You can test: presence of anti-PF4 heparin complex antibodies –> can do via ELISA
6
Q
HITS rx
A
- Cease heparin
- Argatroban: treatment of HITS, is a direct thrombin inhibitor
- Also effective: danaparoid (Xa inhibitor), fondaparinux and bivalirudin
- need anticoagulation for 3 months with non-heparin agent
- Vitamin K blockers are contraindicated: increased risk of developing venous limb gangrene secondary to decreased Protein C
7
Q
Essential thrombocytosis epi
A
- PCV and essential thrombocytosis are more common in women
- > 50 years, incidence increases exponentially after 60 years
- have an increased CVS mortality risk
8
Q
Essential thrombocytosis pathogenesis
A
- essential thrombocytosis followed by PCV more likely to develop
- JAK mutation present in 90-95% of PCV patients, 50-70% of essential thrombocytosis and primary myelofibrosis
- MPL and CALR more commonly in essential thrombocytosis and primary myelofibrosis
- In these disorders, platelets can function abnormally –> occlusion or haemorrhage
- When platelets 400-1000 then thromboembolism is common
- When platelets 1000-2000 then bleeding is more common
- Acquired von Willebrand occurs when the platelet count is >1000
- When the platelet count is within a normal range of a myeloproliferative neoplasm, the risk of thrombosis is uncertain
9
Q
Essential thrombocytosis clinical
A
- Cutaneous in 20% of patients
- Purpura
- Haematomas
- Livedo reticularis
- Erythromelalgia
- Intense burning and paroxysmal bright erythema of the distal extremities
- can occur with any myeloproliferation or myelodysplastic disorder if the platelets are high enough
- platelet mediated acral vasodilation, inflammation and microvascular occlusion
- secondary responds well to aspirin
- Raynaud
- Urticaria
- Vasculitis
- Leg ulcers
- Gangrene
- Superficial thrombophlebitis
- CML often has elevated neutrophil counts, eosinophil or basophil counts
10
Q
Essential thrombocytosis histo
A
- microvascular occlusion of dermal vessels or subcutaneous arterioles
11
Q
Essential thrombocytosis rx
A
- Can give low dose aspirin
- For high risk - >65 with PV or >60 with ET and thrombosis –> low dose aspirin plus cytoreductive therapy
- refractory prutiis in PCV –> paroxetine, interferon alpha, UVB phototherapy, JAK inhibitor
- Imetelstat
12
Q
Paroxysmal Nocturnal Haemoglobinuria pathogenesis
A
- Mutation ins PIG-A in haemotopoietic stem cells - encodes proteins which are responsible for protecting blood cells and platelets from complement mediated injury.
- Lack of protection: red cell lysis and activation of platelets
- X-linked
- Cause of venous thrombosis is multifactorial, due to microparticle formation from platelets and other blood componenet membranes
13
Q
Paroxysmal Nocturnal Haemoglobinuria clinical
A
- Intravascular haemolysis, deficient haematopoiesis and thrombotic tendency
- can occur with aplastic anaemia, myelodysplastic syndrome
- Thrombotic events the most common cause of death, and venous thromboses more common than arterial
- Cutaneous:
- Haemorrhagic bullae
- Petechiae
- Leg ulcers
- Non-inflammatory retiform purpura
- Purpura fulminans like presentation
- Extra-cutaneous: smooth muscle dystonia with dysphagia, abdominal pain and erectile dysfunction, ++ chronic kidney disease
14
Q
Paroxysmal Nocturnal Haemoglobinuria histology
A
- microvascular occlusion
- detection of an absence or severe deficiency of GPI-anchored protein in >2 cell lineages (WCC, RBC) by flow cytometry of peripheral blood is diagnostic of PNH
15
Q
Paroxysmal Nocturnal Haemoglobinuria ddx
A
- haemolytic anaemia + pancytopaenia should suggest PNH
- the ddx is super broad
16
Q
Paroxysmal Nocturnal Haemoglobinuria rx
A
- Eculizumab: inhibits terminal phase of the complement cascade at the C5 stage, and can reduce intravascular haemolysis and venous thrombosis
- this can increase the risk of meningococcaemia via C5 inhibition, so make sure they get the meningococcal vaccine 2 weeks prior
17
Q
Primary thrombotic microangiopathy characterisation
A
- characterised by microangiopathic haemolytic anaemia + thrombocytopaenia
- used to be TTP and HUS, but there are just lots of types of primary and secondary
18
Q
Primary thrombotic microangiopathy inherited subtypes
A
- ADAMTS13 deficiency - TTP:
- onset in childhood
- recurrent TMA, neurologic or other organ
- Rx: plasma infusion or exchange
- Complement mediation
- 90% of inherited cases
- results in uncontrolled activation of the alternative complement pathway
- TMA + renal injury
- Rx: anti-complement: eculizumab, plasma exchange or infusion
- Metabolism mediated - MMACHC mutation
- homocystinuria and methylmalonic aciduria from mutation
- metabolic abnormalities trigger platelet activation
- Occurs in infancy
- Clinical: developmental delay, hypotonia, pulmonary artery hypertension, CKD with hypertension and proteinuria
- Rx: B12, folinic acid, betaine
- Coagulation mediated
- Mutation in THBD, PLG and DHKE - encode thrombomodulin, plasminogen, etc
- results in prothrombosis
- see in infancy
- AKI
- Rx: plasma infusion
19
Q
Primary thrombotic microangiopathy acquired subtypes
A
- ADAMTs12 deficiency
- from autoantibodies leading to inhition of ADAMTS12 activity
- results in vascular occlusion
- Adults: more common in women and blacks
- TMA with thrombocytopaenia, GI symptoms, focal neurological defects, transient renal injury
- Rx: plasma exchange
- Shiga toxin mediated - HUS
- Enteric infection with a toxin secreting strain of E coli or Shigella
- Early childhood
- Infectious enterocolitis, thrombocytopaenia, renal disease
- Supportive care
- Drug mediated - imnune
- Quinines, quetiapine, gemcitabine, interferon-beta
- Adult hood
- Sudden onset of severe systemic symptoms
- Stop the drug!
- Drug mediated - toxic/dose related
- Multiple drugs: cyclosporin, tacrolimus, VEGF inhibitors
- Gradual onset renal failure
- Complement mediated - 10% of cases acquired
- Antibdoies inhibit complement factor H
- Reduced inhibition or allternate complement pathway factors –> vessel damage
- AKI
- Plasma exchange
20
Q
Primary thrombotic microangiopathy clinical
A
- Fever, petechiae, thrombocytopaenia, microangiopathic haemolytic anaemia (schistocytes or framgented red cells prominent on the peripheral smear)
- Renal disease
- Neurologic: headache and confusion
- Macular petechiae: reflect haemorrhage, but occasionally may be from platelet plugging
- Disease may be subclinical unless triggered by factors such as drugs, pregnancy, AI-CT disease, systemic infections, haematopoietic stem cell transplant, malignant hypertension
21
Q
Primary thrombotic microangiopathy histo
A
microvascular occlusion of the visceral terminal arterioles and capillaries
22
Q
Primary thrombotic microangiopathy Ddx
A
- sepsis syndromes
- DIC
- normal or minimally elevated prothrombin time plus profound thrombocytopaenia strongly favours TMA over DIC
- antiphospholipid antibody syndrome
23
Q
What are cryoglobulins
A
immunoglobulins that are present in both serum and plasma, and reversibly precipitate with cold exposure
24
Q
What are cryofibrinogens
A
fibrinogens which precipitate in the cold, are consumed in clotting and therefore detectable only in plasma samples
25
Q
What are cold agglutinins
A
antibodies which promote agglutination of red cells on exposure to cold
26
Q
Disorders of Cryoprecipitation or Cryagglutination pathogenesis
A
- cryoglobulins become water insoluble on exposure to the cold –> increased viscosity
- Type 1: occlusion
- Type 2 & 3: immune complex mediated vasculitis –> inflammatory purpura which is palpable
- When cryoproteins precipitate it results in retiform purpura/necrosis –> reflection of monoclonal immunoglobulins –> type 1 from an underlying plasma cell dyscrasia
- Immune complex disease results in inflammatory purpura that is palpable –> usually due to Type 2 or 3, due to HCV
27
Q
Disorders of Cryoprecipitation or Cryagglutination epidemiology
A
- Cryofibrinogenaemia varies from 0-7% in healthy, and 8-13% in hospitalized
- Female: male is 1.5-4.5:1
- can be secondary to lots of things: carcinomas, infectious, autoimmune
28
Q
Disorders of Cryoprecipitation or Cryagglutination clinical
A
- Cardinal sign: purpura or necrotic lesions, often retiform, at acral sites of cold exposure
- Other cutaneous:
- acral cyanosis
- Raynaud phenomenon
- livedo reticularis
- Type 1 may have secondary vasculitis
- Type 2 may cause occlusion if they are unstable at close to body temperature
- With cryofibrinogenaemia –> need clinical correlation as its actually quite prevalent.
- Cold agglutinins are often asymptomatic, incidental findings such as mycoplasmal infections
- when symptomatic: haemolysis post cold exposure
- very rarely, cold exposure can result in red cell agglutination in cold-exposed sites
- can occur at infusion sites
29
Q
Disorders of Cryoprecipitation or Cryagglutination histo
A
- eosinophilic hyaline occlusion of the blood vessels
30
Q
Disorders of Cryoprecipitation or Cryagglutination ddx
A
- syndromes of distal occlusion: cholesterol emboli, acral antiphospholipid antibody
- Pernio
31
Q
Disorders of Cryoprecipitation or Cryagglutination rx
A
- minimise cold exposure
- Cryoglobulinaemia:
- control underlying plasma cell dyscrasia or lymphoproliferative disorder
- short term: plasmapheresis or plasma exchange
- Cryofibrinogenaemia:
- low dose aspirin and steroids
- Stanozolol
- Immunosuppressants, plasmapharesis, IV fibrinolysis
- Thrombotic: anticoagulation
- Cold agglutinin:
- cold avoidance
- Rituximab - 50% response rate
32
Q
Cholesterol emboli epi
A
- Mean >50 years
- Comorbidities: diabetes mellitus, hyperlipidaemia, hypertension, tobacco use, peripheral artery diease
33
Q
Cholesterol emboli pathogenesis
A
- severe atherosclerotic disease
- three settings to fragment cholesterol:
- Arterial or coronary catheterization
- Prolonged anticoagulation: slowly lyses the clot, leaving exposed areas of the plaque subject to shear stress of arterial flow. Occurs 1-2 months after starting the therapy - ‘warfarin blue toe syndrome’
- Acute thrombolytic therapy - given for MI or stroke, can occur within hours to days
- 20% will have no known inciting event
34
Q
Cholesterol emboli clinical
A
- Systemic: fever, weight loss, myalgias, altered mental status, sudden onset of arterial hypertension
- Cx: embolic –> TIA, strokes, renal failures, GI ulcerations, haemorrhagic pancreatitis
- Cutaneous:
- Livedo reticualris
- Cyanosis/blue toes - 30-75%
- Ulceration - 15-40%
- Peripheral gangrene
- Nodules
- Purpura
- Can get upper extremity lesions can occur if the plaque originated in the aortic arch
- Initial diagnosis made in only 35-40% of patients
- Ix: eosinophilia, hypocomplementaemia, leukocytosis, elevated ESR, CRP, serum creatinine, BUN, amylase
- Urine: pyuria, eosinophiluria, heme positive urine or stool
35
Q
Cholesterol emboli histo
A
- Cholesterol emboli: elongated clefts within lumina of small vessels –> clefts are from dissolution of cholesterol crystals during the fixation process
- neutrophils, eosinophils and mononuclear cells in the arterial walls within 24-48 hours, multinucleated histiocytes within 3-6 days (make sense)
36
Q
Cholesterol emboli ddx
A
- abrupt onset should worry those about cholesterol or oxalate embolism
- retiform purpura, especially when it occurs distally in concert with extensive distal livedo reticularis, is especially suggestive of this diagnosis
- Acral cold occlusion syndromes need to be considered
37
Q
Cholesterol emboli rx
A
- supportive, if possible surgical bypass or endovascular stent grafting
- aspirin, antiplatelets, statins, discontinuation of anticoagulation
- severe renal damage –> initiation of anticoagulation
- systemic steroids
- iloprost
- hyperbaric oxygen
38
Q
Oxalate Embolus path and epi
A
- Really rare, occurs in association with primary hyperoxaluria
- rare enzyme deficiency
- increased oxalaic acid accumulation
39
Q
Oxalate Embolus clinical
A
- Childhood: urolithiasis –> deposits in the kidney –> renal failure
- Once renal failure occurs, then embolus usually occurs
- death by age of 20 is not uncommon
- Secondary or acquired may result from taking oxalate precursors, increased absorption, pyridoxine deficiency, impaired renal excretion or bowel disease (Crohns)
- Cutaneous:
- acrocyanosis
- Raynaud
- peripheral gangrene
- acute cutaneous necrosis
- livedo reticularis
- erythematous ulcerated nodules
- miliary calcified deposites
- cutaneous calciphylaxis like presentation –> can be confusing because also renal failure
40
Q
Oxalate Embolus histo
A
- yellow-brown birefringent crystals in a rectangular or radial pattern within and around vessels in the deep dermis and subcutaneous tissue
- crystals are yellow to golden brown in routinely stained sections, and birefringent under polarized light
- stain with alizarin red at pH 7
- no calcium deposition
41
Q
Oxalate Embolus ddx
A
- cholesterol embolus
- cutaneous calciphylaxis
- other retiform purpura and livedo reticularis
42
Q
Oxalate Embolus rx
A
- Type 1 primary hyperoxaluria without renal failure: oral pyridoxine
- Type 2: hydration and alkalinization of the urine
- no point doing a transplant without fixing the underlying issue
- hepatic transplant may reverse it
43
Q
Atrial myxoma
A
- are, 3rd - 6th decade of life. Constitutional symptoms: fever, malaise, arthralgia, weight loss, blood flow obstruction, embolic phenomena
- Cutaneous: acral papules, purpuric, serpiginous or annular violaceous fingertip lesions, splinter haemorrhages, livedo reticularis, Raynaud, toe necrosis, malar flush, distal petechiae
- Carney complex: multiple early onset cardiac myxomas
44
Q
Neonatal Purpura Fulminans
A
- Homozygous deficiency or severe dysfunction of protein C or protein S leads to neonatal purpura fulminans
- Its interesting because if they have deficiency of antithrombin 3 then they get DVTs and PEs but minimal cutaneous findings
- Clinical: non-inflammatory retiform purpura –> large scale cutaneous and limb necrosis, and visceral organ involvement
- Often born with cerebral thrombosis or retinal vessel occlusion with congenital blindness
- Can get acquired from group B strep infections
- Rx: intravenous protein C or fresh frozen plasma as initial treatment, then warfarin or LMWH, followed by tapering of factor replacement. they are then anticoagulated for life
45
Q
Warfarin necrosis
A
- Abnormal gamma-carboxylation by the liver of vitamin K sensitive factors –> 2, 7, 9, 10, protein C and, could also get from severe vitamin K deficiency
- shortest half lives are factor 7 and protein C –> factor 7 is needed to switch to procoagulation
- so in warfarin necrosis, the procoagulant system takes longer to reach its low point equilibrium than protein C dependant anticoagulation does
- Starts 2-5 days after starting warfarin without heparin, more likely to occur if loading doses occur, and is when the protein C drops
- 4X higher in women, peak incidence 60-70s
- late onset warfarin necrosis is also a thing, for people with poor compliance or inappropriate dosing, liver function alteration or drug interactions
- Clinically:
- areas with lots of subcut fat: breast, hip, buttock or thigh
- pain, followed by well-demarcated erythema –> rapidly becomes haemorrhagic and necrotic
- partial retiform or branching purpura typically can be seen within or at the margin of the cutaneous lesions
- Biopsy: thrombosis of most of the dermal vessels
- Rx: stop warfarin, start vitamin K and heparin, and protein C replacement
46
Q
Warfarin associated venous limb gangrene
A
- malignancy and HITs –> venous limb ischaemia and gangrene
- malignancy –> platelet consumption and coagulopathy
- INR is elevated as well
- persistent thrombin generation
47
Q
Purpura fulminans with sepsis
A
- Meningococall infection: meningococcus’ ability to bind directly to endothelial receptors via its type 4 pili
- Can also occur with S aureus, HIb and a few others
- In bubs most commonly from GBS
- Septic patient with DIC:
- retiform purpura can be the first sign –> inability of protein C-thrombodulin pathway to prevent clot propagation
48
Q
Purpura fuliminans post infection
A
- Children post group A strep, varicella zoster or HHV6 –> acquire and antibody that inhibits protein S function
- occurs 7-10 days after initial infection
- Rx: would ideally like to treat with protein S, but there is no such thing really, so trial plasma exchange, plasmapharesis, plasma replacement + LMWH and sometimes IVIG
49
Q
Antiphospholipid Antibody/ Lupus Anticoagulant Syndrome Diagnosis
A
Diagnosis - Sapporo-Sydney Criteria
You need at least 1 clinical and 1 lab criteria:
Clinical:
1. Vascular thrombosis –> can occur anywhere
2. Pregnancy morbidity
1. 1 or more unexplained foetal death after 10 weeks
2. Or 1 or more premature births of morphologically normal neonates before 34 weeks due to eclampsia/pre-eclampsia/placental insufficiency
3. 3 ore more spontaneous abortions
Lab criteria:
1. Anti-cardiolipin antibodies, IgM or IgG
2. Lupus anticoagulant
3. Anti beta-2 glycoprotein
50
Q
Antiphospholipid Antibody/ Lupus Anticoagulant Syndrome epidemiology
A
- Females ++
- mean age 42 +/- 14 years
- Primary: 53%, secondary lupus 36%, lupus like sydnromes 5%
- Patients with lupus: more episodes of arthritis, livedo reticularis, thrombocytopaenia, leukopenia
- Females more likely to have arthritis, livedo reticularis, migraines
- Men: MI, epilepsy, arterial thrombosis
51
Q
Antiphospholipid Antibody/ Lupus Anticoagulant Syndrome pathogenesis
A
multiple mechanisms for anti-phospholipid antibodies mediating thrombosis –> interference with release of prostacyclin, protein C and S pathways, activation of platelets by interacting with platelet membrane phospholipids, etc
52
Q
Antiphospholipid Antibody/ Lupus Anticoagulant Syndrome clinical
A
- Cutaneous:
- Livedo reticularis - 24%
- Leg ulcers 5.5%
- Pseudovasculitic lesions 3.9%
- Digital gangrene
- Cutaneous necroiss
- Splinter haemorrhages
- Acral livedo reticularis
- Livedoid vasculopathy or Degos-like lesions
- Anetoderma like lesions with microthrombosis
- Raynaud
- Behcet like
- PG like
- Nail fold ulcers
- Pseudo-Kaposi sarcoma
- Extra-cutaneous
- DVT/PE
- CNS
- Catatrophic APLS - precipitated by surgery, drugs, discontinuation of anticoagulants, infections
- Also quite possibly can have seronegative group
53
Q
Antiphospholipid Antibody/ Lupus Anticoagulant Syndrome path and lab investigations
A
- Antiphospholipid antibodies and lupus anticoagulants are detected by different assays - if positive for both its because they actually probably are
- Lupus anticoagulant
- most specific
- poorly predictive of thrombosis
- Anti-cardiolipin antibodies
- more positive than lupus
- specificity for clinically significant disease is lower
- Histo:
- non-inflammatory thrombosis of small dermal vessels
54
Q
Antiphospholipid Antibody/ Lupus Anticoagulant Syndrome ddx
A
- in lupus, APLS is particularly likely to present as persistent moderate to extensive livedo reticularis or as livedoid vasculopathy
- Catastrophic may mimic DIC or ADAMTS13 deficiency
- Levamisole adulterated cocaine: neutropenia, aPL antibodies, ANCA
55
Q
Antiphospholipid Antibody/ Lupus Anticoagulant Syndrome rx
A
- Heparin and chronic anticoagulation
- Aspirin for arterial events or stroke
- Lupus: antimalarial
- Catastrophic: systemic steroids, plasma exchange, IVIG, ritux, cyclophosphamide, eculizumab
56
Q
Sneddon syndrome epi
A
- F>M
- third or fourth decade of life
57
Q
Sneddon syndrome pathogenesis
A
- often considered a manifestation of APLS, but not always positive antibodies
- Distinctive vasculopathy of smaller arteries and larger arterioles - particularly skin and brain
- Significant overlap with autosomal recessive disorder ADA2 deficiency
58
Q
Sneddon syndrome clinical
A
- persistent, widespread livedo racemosa –> can precede neuro for many years
- labile hypertension
- CNS disease
- Hx foetal loss
- Raynauds
- those who are aPL antibody negative are less likely to have seizures, mitral regurg, thrombocytopaenia but more likely to have larger pattern livedo reticularis
59
Q
Sneddon syndrome histo
A
- take the biopsy from normal appearing skin the centre of livedo reticularis
- endothelial inflammation, subendothelial myointimal hyperplasia, occlusion (partial or whole) of involved arterioles
60
Q
Sneddon syndrome ddx
A
- Other livedo racemosa ddx
- ADA2 deficiency –> paeds
- make sure to test aPL antibodies
61
Q
Sneddon syndrome rx
A
- Warfarin, although not routinely effective
- Systemic steroids or immunosuppressants not helpful either
- in those who are aPL negative, antiplatelets were just as effective so use aspirin/clopidogrel
62
Q
Livedoid vasculopathy epi
A
- young to middle aged women
- can be divided into:
- Primary
- Secondary –> associated with varicosities, etc
63
Q
Livedoid vasculopathy pathogenesis
A
- unknown, believed to be alteration in local or systemic control of coagulation with formation of fibrin thrombi focially wihtin superficial dermal blood vessels
- number of prothrombotic factors has been associated with this syndrome lijke protein C and S, factor 5 leiden, etc
64
Q
Livedoid vasculopathy clinical
A
- painful, persistent and often punched-out ulcerations on the legs –> particularly the malleoli in women
- some patients may develop retiform or stellate purpura or ulcer extension
- heals with white atrophic scars with peripheral telangiectasias
- 50% –> associated prothrombotic abnormality has been noted
65
Q
Livedoid vasculopathy ddx
A
Antiphospholipid antibody syndrome
atrophie blanche like lesions: APLS, vasculitis, sickle cell disease, hydroxyurea related leg ulcers
66
Q
Livedoid vasculopathy histo
A
- mild peri-vascular lymphocytic infiltrate
- extravasated red cells surrounding superficial dermal vessels with hyalinized walls and luminal fibrin deposition
- IF non-specific: IgM, C3 may be within vessel walls
67
Q
Livedoid vasculopathy rx
A
- anticoagulants as monotherapy
- other options: anabolic steroids, IVIG, antiplatelets
68
Q
Malignant atrophic papulosis epi
A
- females, 20s-40s
- can be familial
69
Q
Malignant atrophic papulosis pathogenesis
A
- small vessel vasculopathy –> vaso-occlusive disorder
- several diseases can mimic it: APLS, lupus, DM
- Two forms:
- Systemic - dysregulation of interferon alpha and C5-9 membrane attack complex
- Benign - skin limited
70
Q
Malignant atrophic papulosis clinical
A
- Cutaneous:
- crops of small 2-5 mm erythematous papules on the trunk or extremtiies, these evolve over 2-4 weeks with central depression
- porcelain white scar often with a rim of telangiectasias and appearance similar to atrophie blanche
- typically precede systemic manifestations
- Gastrointestinal: bowl perforation
- CNS manifestations
- Probability of having benign form of disease 70%, after 7 years of skin limited disease then icnreases to 97%
71
Q
Malignant atrophic papulosis histo
A
- Wedge shaped area of ischaemic dermis with a sparse perivascular lymphohistiocytic infiltrate at the edge of the ischaemic area
- atrophic but slightly hyperkeratotic overlying epidermis
- Oedema and mucin in the ischaemic dermis
- Late stage sclerosis may be seen
- base of lesion: vascular damage with thrombosis
72
Q
Malignant atrophic papulosis ddx
A
- lupus, DM
- APLS
73
Q
Malignant atrophic papulosis rx
A
- no proven treatment for idiopathic
- Aspirin, dipyridamole, eculizumab (decreases C5-9 membrane attack complex deposition) and trepostinil
- Mixed results with IVIG
